ArticleHuman molecular genetics2014
Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.
Article in Human molecular genetics, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 64 papers.
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Who cites it
64 citing papers in PubMed, 101 citations in OpenAlex.
- Compartment-Specific Mitochondrial Proteomic Alterations in Rat Hippocampus Following Chronic Social Isolation Stress.International journal of molecular sciences · 2026Review
- Multi-omics Analysis Implicates Mitochondrial Complex Assembly Protein COX18 in Mitochondrial Signaling and Tumorigenesis across Cancers.Cell biochemistry and biophysics · 2025Article
- COA5 has an essential role in the early stage of mitochondrial complex IV assembly.Life science alliance · 2025Article
- Nuclear Control of Mitochondrial Homeostasis and Venetoclax Efficacy in AML via COX4I1.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- Prenatal Counseling and Diagnosis of COX20 Gene-Related Mitochondrial Complex IV Deficiency: A Case Report and Literature Review.International journal of women's health · 2025Article
- Structural basis of LRPPRC-SLIRP-dependent translation by the mitoribosome.Nature structural & molecular biology · 2024Article
- A dual role for PSIP1/LEDGF in T cell acute lymphoblastic leukemia.Science advances · 2024Article
- Article
- Identification of fatty acid metabolism signature genes in patients with pulmonary arterial hypertension using WGCNA and machine learning.The Journal of international medical research · 2024Article
- Identification of factors limiting the allotopic production of the Cox2 subunit of yeast cytochrome c oxidase.Genetics · 2024Article
- More than Just Bread and Wine: Using Yeast to Understand Inherited Cytochrome Oxidase Deficiencies in Humans.International journal of molecular sciences · 2024Review
- Identification of TMEM126A as OXA1L-interacting protein reveals cotranslational quality control in mitochondria.Molecular cell · 2024Article
- BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome.Nucleic acids research · 2023Article
- Two-FoldAnimals : an open access journal from MDPI · 2023Article
- Human Tim8a, Tim8b and Tim13 are auxiliary assembly factors of mature Complex IV.EMBO reports · 2023Article
- Tetratricopeptide-containing SMALL KERNEL 11 is essential for the assembly of cytochrome c oxidase in maize mitochondria.Plant physiology · 2023Article
- Clinical and genetic characteristics of children with COX20-associated mitochondrial disorder: case report and literature review.BMC medical genomics · 2023Review
- Genomics of Secondarily Temperate Adaptation in the Only Non-Antarctic Icefish.Molecular biology and evolution · 2023Article
- Whole-exome identifies germline variants in families with obstructive sleep apnea syndrome.Frontiers in genetics · 2023Article
- Tissue-specific mitochondrial HIGD1C promotes oxygen sensitivity in carotid body chemoreceptors.eLife · 2022Article
4 more citing papers are in PubMed but not listed here.
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
Abstract
Cytochrome c oxidase (CIV) deficiency is one of the most common respiratory chain defects in patients presenting with mitochondrial encephalocardiomyopathies. CIV biogenesis is complicated by the dual genetic origin of its structural subunits, and assembly of a functional holoenzyme complex requires a large number of nucleus-encoded assembly factors. In general, the functions of these assembly factors remain poorly understood, and mechanistic investigations of human CIV biogenesis have been limited by the availability of model cell lines. Here, we have used small interference RNA and transcription activator-like effector nucleases (TALENs) technology to create knockdown and knockout human cell lines, respectively, to study the function of the CIV assembly factor COX20 (FAM36A). These cell lines exhibit a severe, isolated CIV deficiency due to instability of COX2, a mitochondrion-encoded CIV subunit. Mitochondria lacking COX20 accumulate CIV subassemblies containing COX1 and COX4, similar to those detected in fibroblasts from patients carrying mutations in the COX2 copper chaperones SCO1 and SCO2. These results imply that in the absence of COX20, COX2 is inefficiently incorporated into early CIV subassemblies. Immunoprecipitation assays using a stable COX20 knockout cell line expressing functional COX20-FLAG allowed us to identify an interaction between COX20 and newly synthesized COX2. Additionally, we show that SCO1 and SCO2 act on COX20-bound COX2. We propose that COX20 acts as a chaperone in the early steps of COX2 maturation, stabilizing the newly synthesized protein and presenting COX2 to its metallochaperone module, which in turn facilitates the incorporation of mature COX2 into the CIV assembly line.
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